aortic arch in eisenmenger`s complex

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Thorax (1958), 13, 272.

ABSENT LEFT PULMONARY ARTERY AND RIGHT-SIDED AORTIC ARCH IN EISENMENGER'S COMPLEX BY

H. A. FLEMING From the Cardiac Department, Brompton Hospital, London (RECEIVED FOR PUBLICATION JANUARY 6, 1958)

Absence of a pulmonary artery is relatively rare, and when McKim and Wiglesworth reported six examples in 1954 they could find only 11 others in the literature since 1868. Most of them had been discovered at operation or necropsy. As Maier (1954) pointed out, the anomaly is found much more frequently in patients submitted to angiocardiography than at necropsy, when it can easily be ov erlooked. By 1956 Emanuel and Pattinson were able to find 46 cases reported in the literature. Of these, 18 were associated with Fallot's tetralogy, and in all but one it was the left pulmonary artery which was absent; in the exception, there was dextrocardia and situs inversus. The absence of the left pulmonary artery was associated with a right-sided aorta in 60% of these cases. Most of the other cases that have been reported have also been associated with a major cardiac or vascular defect, although Maier (1954) mentioned one in which no other anomaly could be found. Wyman (1954) likewise reported a case in which absence of the pulmonary artery was the sole defect, and Steinberg, Dotter, and Lukas (1953) described two others in patients aged 26 and 53 years. No associated congenital abnormalities in other systems have been cited. Nadas, Rosenbaum, Wittenborg, and Rudolph (1953) stated that all cases of absent pulmonary artery without a cardiac defect occurred on the right side; one of the two cases described by Steinberg and others (1953), however, had an absent left pulmonary artery. McKim and Wiglesworth (1954) noted that the acwtic arch commonly lies on the side opposite to the absent pulmonary artery. Although studies of associated cardiac lesions have often been incomplete, there is evidence that Fallot's tetralogy is particularly common with this abnormality, a point that should be borne in mind when contemplating Blalock's anastomosis (Nadas and others, 1953). Other associated lesions so far

reported include atrial septal defect, patent ductus arteriosus, coarctation of the aorta, pulmonary stenosis, absence of the transverse aortic arch, and persistent truncus arteriosus (Humphreys, 1932; Sweet and White, 1950; Kjellberg, Mannheimer, Rudhe, and Jonsson, 1955). Eisenmenger's complex has been recorded only once, in a necropsy on a 20-months-old boy with an absent left pulmonary artery and a right-sided aortic arch (McKim and Wiglesworth, 1954). The purpose of the present paper is to present a case of Eisenmenger's complex with absent left pulmonary artery and right-sided aortic arch in which the diagnosis was established during life. This is believed to be the first such case reported. CASE REPORT The patient was a little girl aged 7 years, from Turkey. Since she was not accompanied by her parents or anyone speaking English, it was not possible to obtain a complete history. Cyanosis dated from birth and was increasing; it was particularly noticeable when she was tired or when she exerted herself. There was a dubious history of precordial discomfort on exertion. In the ward she was observed to squat very frequently and she tired easily. She was small for her age, being 3 ft. 7j in. (110 cm.) in height and weighing 35 lb. (16 kg.). The sternum protruded markedly, but she had no other external congenital abnormalities. There was moderate central cyanosis and clubbing of the fingers and toes. All peripheral pulses were normal. The blood pressure was 120/80 mm. Hg in both arms. The jugular venous pressure was not raised, but the a wave was abnormally conspicuous. The apex beat was displaced to the left, being formed by a hyperdynamic right ventricle felt in the anterior axillary line. The pulmonary artery impulse was impalpable. On auscultation there was a loud right ventricular third heart sound, increasing on inspiration. In the second left intercostal space was a loud systolic click preceding a short systolic ejection murmur. The

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